Many patients with PHG also have putative immune disorders such as sclerosing mediastinitis, i.a.s retroperitoneal fibrosis'" and Riedel's thyroiditis," Staining and culture evaluations for the presence ofbacteria and fungus generally yield negative results, although a positive Histoplasma stain was reported in one case. 8 Up to 25 percent of patients with PHG have no symptoms; others have fever, malaise, weight loss, cough, dyspnea, occasional hemoptysis and chest pain. 1.3.' The disease affects mainly young and middle-aged persons-the youngest patient described was 19-and occurs at about the same rate in both sexes and all races. On roentgenographic evaluation, PHG appears as weUdefined rounded or slightly irregular nodules.P Cavitation I.? and calcification3 •5 rarely are observed. Solitary nodules, infiltrates and consolidation have been seen. 1.3 Our patient with PHG and cavitary calcified nodules had a weakly positive test for antinuclear antibodies, a slight elevation in complement levels and hyperglobulinemia. Although staining and culture assessments of an excised nodule were negative for Histoplasma, the existence of a Histoplasma infection was documented by the presence of calcified splenic granulomas and positive results on a histoplasmin skin test. The pathogenesis of PHG is unclear, but an infection with M tuberculosis, Histoplasma or other antigenic agents may trigger development of the disease.'> Yousem and Hochholzer- exclude the diagnosis of PHG if hilar or mediastinal calcification is present, but a nodule with pathologic characteristics of PHG has been found in a patient with histoplasmosis with sclerosing mediastinitis and subcarinal calcification." In our patient, therefore, an infection with Histoplasma probably triggered a hyperimmune reaction that resulted in PHG. ACKNOWLEDGMENTS: We thank Renee J. Robillard for editorial assistance and Erika L. Whitmore, M.D., for performing the pathologic studies. REFERENCES
1 Engleman P, Liebow AA, Gmelich J, Friedman PI. Pulmonary hyalinizing granuloma. Am Rev Respir Dis 1977; 115:997-1008 2 Joseph MG, Colby Tv, Swensen SJ, Mikus JP, Gaensler EA. Multiple cystic fibrohistiocystic tumors of the lung: report of two cases. MayoClin Proc 1990;65:192-97 3 Yousem SA, Hochholzer L. Pulmonary hyalinizing granuloma. AmJ Coo Patholl987; 87:1-6 4 Scholsnagle DC, Check IJ, SeweD Cvv, Plummer A, York RM, Hunter RL. Immunologic abnormalities in two patients with pulmonary hyalinizing granuloma. Am J Clin Pathol 1982; 78:231-35 5 Dent RG, Gooden OJ, Stavin PCI, Stark JE. Pulmonary hyalinising granuloma in association with retroperitoneal fibrosis.Thorax 1983; 38:955-56 6 Guccion JG, Rohatgi PK, Saini N. Pulmonary hyalinizing granuloma, electron microscopic and immunologic studies. Chest 1984; 85:571-73 7 Gans SJM, van der Elst AMC, Straks vv. Pulmonaryhyalinizing granuloma. Eur RespirJ 1988; 1:389-91 8 Chalaoui J, Gregoire P, Sylvestre J, Lefebvre R, Amyot R. Pulmonaryhyalinizing granuloma: a causeofpulmonarynodules. Radiology 1984;152:23-26 9 Ikard Rvv. Roentgenogram of the month: pulmonary hyalinizing granuloma. Chest 1988;93:871-72 10 Case records of the Massachusetts General Hospital (case 61989). N Engl J Med 1989;320:380-89
Angioimmunoblastic Lymphadenopathy Presenting as SUPerior Vena Caval Obstruction* SarUe8V Sanghvi, M.D.;t Ajeet S. Kothari, M.D.;:j: Bhushan C. Hathi, MB, BS.;§ and Raj G. Sharma, M.S.lI
A 60-year-old man presented with features of superior vena cava (SVC) obstruction. On evaluation, he was diagnosed as having angioimmullQblastic lymphadenopathy with dysproteinemia (AILD). SVC obstruction due to AILD, to our knowledge, has not been described. (Chest 1991; 100:1721-22)
=
AILD angioimmunoblastic lymp.hadenopathy with dysproteinemia; SVC= superior vena cava
.l ngioimmunoblastic lymphadenopathy with dysproteine.l'1 mia (AlLD) is an established clinicopathologic entity with a characteristic histologic picture that differentiates it from lymphoma.!" AlLD commonly presents as generalized lymphadenopathy, hepatosplenomegaly, and constitutional symptoms, ... but skin rashes and pulmonary involvement have been noted in several patients.' Occasionally, it may present with localized lymphadenopathy, Hashimoto's thyroiditis, cryoglobulinemia, and carcinoma of the pancreas.• To our knowledge, superior vena cava (SVC) obstruction as a presenting manifestation of AILD has so far not been reported. We report a patient with AILD who presented with SVC obstruction. CASE REpORT
A 60-year-old man presented with complaints of puffiness of the
face, irregular fever, headache, cough, breathlessness, and gener-
alized weaknessfor three months. There was no history of hemoptysis, orthopnea, dysphagia, urinary complaints, weight loss, skin rash, or drug ingestion. The patient used to smoke 20 cigarettes per day for the past 20 years. On examination, the face was suffused, conjunctival congestion was present, right supraclavicular, right submandibular, and bilateral axillary lymph nodes were enlarged; neck veinswere engorged, prominent veins were visible over the anterior chest wall and abdominalwallwith direction of blood flow from abovedownwards. Liver and spleen were just palpable. Results of examination of the respiratory system were normal except for a positive D'Espines sign. Other systems did not reveal any abnormality. Laboratory findings were as foDows: hemoglobin, 9 Wdl; ESR, 55 mm in first hour; total and differential leukocyte counts, peripheral blood film,and serum biochemistrywere normal;serum total protein, 6.5 g/dl, A:G ratio, 1:1.5; IgG, 1.4 Wdl; 19A, 0.35 Wdl; and IgM, 0.85 g/dl. Coombs' test was negative. Bone marrow study was normal. Sputum was negative for malignant cells and acid-fast bacilli. Skiagram of chest showed obliterilion of right costophrenic angle, with mediastinal and hilar adenopathy (Fig 1). Barium swallow study was normal. Histopathologic examination of lymph nodes showed partial effacement of lymph node architecture with proliferationof blood *From the M. G. Hospital and Dr. S. N. MedicalCollege, Jodhpur (Rajasthan) India. tAssistant Professorof Medicine. :j:Medical Officer, Medicine. §Registrar, Medicine. 'IlAssistant Professorof Surgery. CHEST I 100 I 6 I DECEMBER, 1991
1721
FIGURE 1. Chest roentgenogram showing mediastinal and hilar adenopathy and obliteration of right costophrenic angle. vessels throughout the node and sheets ofimmunoblasts, histiocytes, and plasma cells, mixed at places with lymphocytes. Occasional eosinophils were also seen (Fig 2). Adiagnosis ofangioimmunoblastic lymphadenopathy was thus made. The patient was given 60 mg of prednisolone per day. Within two weeks, the patient had marked decrease in facial puffiness and venous engorgement, and regression of lymphadenopathy. Then, prednisolone therapy was tapered and discontinued after a period of one month. At eight months of follow-up, the patient continues to be asymptomatic. DISCUSSION
Angioimmunoblastic lymphadenopathy with dysproteinemia is an uncommon disorder that mimics lymphomas but can be differentiated histologically. The characteristic features seen in lymph nodes include proliferation of immunoblast cells and plasma cells, arborizing small blood
FIGURE 2. Histopathologic features of the lymph node showing proliferation of blood vessels, sheets of immunoblasts, histiocytes, plasma cells, and lymphoid cells.
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vessels, interstitial deposition of homogenous eosinophilic material, and obliteration of normal architecture.... The presence of these features in our patient clinched the diagnosis of AlLD. The etiology and pathogenesis of AlLD is still unknown. >,3,' The clinical, laboratory, and patholOgiC manifestations are suggestive of an autoimmune disorder in which abnormalities of T cells are a consistent feature which, probably, predispose to abnormal B-cell proliferation leading to hypergammaglobulinemia.... It has also been reported to develop following ingestion of drugs such as antibiotics, oral antidiabetics, hydantoin, and thiazides.· Similarities with immunoproliferative syndromes, systemic lupus erythematosus, graft vs host disease, and acquired immunodeficiency syndrome have been described.>,' Clinically, AlLD usually presents as generalized lymphadenopathy with hepatosplenomegaly and constitutional symptoms. Presentation as SVC obstruction due to mediastinal lymph node enlargement, though not unpredictable, has not been reported previously in AILD (to our knowledge). Our patient presented with classic features of SVC obstruction. This is a well-recognized complication of many mediastinal conditions. The vast majority of cases are due to malignant disorders, especially bronchogenic carcinoma and lymphoma. Metastasis from breast, germ cell carcinoma, mesothelioma, and thymoma uncommonly produce SVC obstruction. About 10 to 25 percent of cases have been reported in various series to have benign causes such as mediastinal fibrosis, histoplasmosis, thrombosis, goiter, aneurysm, and radiation fibrosis! Depending on the course of the disease, two types of AlLD have been recognized. On one hand, long survival has been observed, while on the other, patients have died in less than 1.5 years regardless of the treatment received.> The appropriate therapy for AlLD is not known. Frizzera et a1> suggested symptomatic treatment with observation of the patient, as spontaneous regression of the process may occur. Recommendations for therapy have ranged from no treatment to aggressive multidrog chemotherapy, but supportive therapy and small doses of steroids appear to be a safe therapy. Our patient responded well to this approach. REFERENCES
1 Frizzera G, Moran EM, Rappaport H. Angioimmunoblastic lymphadenopathy with dysproteinemia. Lancet 1974; 1:1070-73 2 Frizzera G, Moran EM, Rappaport H. Angioimmunoblastic lymphadenopathy: diagnosis and clinical course. Am J Med 1975; 59:803-18 3 Lukes RJ, TIndle BH. Immunoblastic lymphadenopathy: a hyperimmune entity resembling Hodgkin's disease. N Engl J Med 1975; 292:1-8 4 Schauer PK, Straus OJ, Bagley CM, Rudolph RH, Mc Cracken JO, Huff J, et al. Angioimmunoblastic lymphadenopathy: clinical spectrum of disease. Cancer 1981; 48:2493-98 5 Pizzolo G, Vinante F, Agostini C, Zambello R, Trentin L, Masciarelli M, et al. Immunologic abnormalities in angioimmunoblastic lymphadenopathy. Cancer 1987; 60:2412-18 6 Makashar R, Singh T, Jayaram G. Angioimmunoblastic lymphadenopathy with dysproteinemia. J Assoc Physicians India 1987; 35:304-05 7 Schraufnagel DE, Hill R, Leech JA, Pare lAP. Superior vena cava obstruction: is it a medical emergency? Am J Med 1981; 70:116974 Anglolmmunobl8stic Lymphadenopa1hy (SBnghvi et aI)